CLN2, Polyclonal Antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Purified by antigen-affinity chromatography.

Supplied in 0.1M Tris-buffered saline with 10% Glycerol (pH7.0). 0.01% Thimerosal was added as a preservative.

Store at -20 degree C for long term preservation (recommended). Store at 4 degree C for short term use.

Small volumes of anti-CLN2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

This gene encodes a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome. [provided by RefSeq]

Total protein Ab

Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

Western blotting: 1:500-1:3000
Immunohistochemistry: 1:100-1:250
Immunofluorescence: 1:100-1:200

Sample (30 ug of whole cell lysate) A: A431 B: H1299 C: Hela 7.5% SDS PAGE Primary antibody diluted at 1: 1000

Immunohistochemical analysis of paraffin-embedded SW480 xenograft, using TPP1 antibody at 1: 100 dilution.

Immunofluorescence analysis of methanol-fixed A431, using TPP1 antibody at 1: 200 dilution.

34,464 Da

tripeptidyl peptidase I

tripeptidyl-peptidase 1

Tripeptidyl-peptidase 1

CLN2; TPP-1; LPIC; TPP-I  [Similar Products]

TPP1_HUMAN

This gene encodes a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome. [provided by RefSeq, Jul 2008]

TPP1: Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus. Defects in TPP1 are the cause of neuronal ceroid lipofuscinosis type 2 (CLN2). A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment pattern seen most often in CLN2 consists of curvilinear profiles. Belongs to the peptidase S53 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; EC 3.4.14.9; Protease; Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 11p15

Cellular Component: lysosomal lumen; mitochondrion; lysosome; melanosome

Molecular Function: peptidase activity; tripeptidyl-peptidase activity; protein binding; serine-type peptidase activity; metal ion binding; serine-type endopeptidase activity; endopeptidase activity; peptide binding

Biological Process: nervous system development; unfolded protein response, activation of signaling protein activity; cellular protein metabolic process; epithelial cell differentiation; lysosome organization and biogenesis; unfolded protein response; peptide catabolic process; protein catabolic process; lipid metabolic process; proteolysis; neuromuscular process controlling balance; bone resorption

Disease: Ceroid Lipofuscinosis, Neuronal, 2

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